Aneurysms of the extracranial carotid artery are rare and may present as mass of tonsil fossa, lateral pharyngeal region and neck, with ischemic symptoms, or with hemorrhage. The causes of this disease include trauma, atherosclerosis, syphilis, mycosis other degenerative or congenital vascular wall conditions. Although physical diagnosis is not difficult, but we often make misdiagnosis due to its rarity. Furthermore degenerative process involving the arterial wall often make surgical repair difficult. Recently authors experienced a case of extracranial carotid artery areurysm, which was successfully removed and reconstructed with saphenous vein graft. So we reviewed the clinical characteristics, possibility of misdiagnosis and problem of management.